胆道闭锁Kasai术后生存分析

目的 探讨胆道闭锁患儿Kasai术后各因素对生存期的影响。方法 对51例胆道闭锁Kasai术后患儿进行回顾性分析,观察生存时间,以Kaplarr—Meier法描述生存曲线,收集可能影响预后的若干因素,运用Cox比例风险模型,进行统计分析。结果 术后胆汁引流量和是否长期随诊,积极治疗影响生存时间。全组3个月、6个月、12个月的累积生存率分别为69．15％、56．33％、32．50％,中位生存期为8个月;手术日龄越大,中位生存期越短。结论 Cox多因素分析认为：术后胆汁引流量和是否长期随访、积极治疗是影响生存时间的相关因素。手术日龄不同,生存曲线有较大变化。     

胆道闭锁Kasai术后类固醇辅助治疗的现状和研究进展

本综述介绍了胆道闭锁Kasai肝门空肠吻合术后的类固醇辅助治疗方案,总结了历年国际及国内范围对于该辅助治疗的临床试验报道,综合对类固醇基础研究的文章,分别从类固醇作用机制、临床应用方案、不良反应及近年循证医学证据等方面进行了详细的描述,在讨论中提出目前仍需实行多个前瞻性临床随机对照试验以重复观察及评价胆道闭锁术后类固醇辅助治疗的利弊.     

胆道闭锁Kasai术后早期并发症的分析

目的 分析胆道闭锁Kasai术后早期并发症与激素抗生素治疗方案、生存预后的关系.方法 回顾性分析1994年8月至2008年8月Ⅲ型胆道闭锁Kasai术后281例临床资料.根据治疗方案分为一般治疗A组和术后大剂量激素和抗生素治疗B组.随访术后2年生存,分析早期并发症与手术日龄、术前胆红素、术后激素抗生素治疗方案、生存预后之间的关系.结果 术后共93例(33.1%)发生早期并发症,急性胆管炎最常见.无并发症与并发症相比,术前总胆红素及手术日龄无差异;危险因素分析,急性胆管炎、上消化道出血分别与术前胆红素及手术日龄无明显相关.B组并发症较A组明显降低(25.7%比63.6%,P＜0.01),其中反流性胆管炎较A组明显降低(23.9%比56.4%,P＜0.01),但术后早期消化道出血无统计学差异.2年随访率92.5%,术后2年以上存活率51.2%,早期并发症2年生存率较无早期并发症明显降低(34.4%比59.6%,P＜0.01).急性胆管炎2年生存率亦明显降低(32.9%比59.2%,P＜0.01).B组2年生存率高于A组(54.0%比40.0%,P=0.043＜0.05).结论 胆道闭锁Kasai术后早期急性胆管炎是影响术后生存的危险因素;术后大剂量应用激素和抗生素疗法明显降低早期急性胆管炎发生率,提高术后2年生存率.

Abstract：

Objective The purpose of this study was to evaluate the relation between early complications of Kasai operation and high-dose steroids and antibiotics and to demonstrate the prognosis of biliary atresia(BA). Methods 281 patients diagnosed with biliary atresia(BA) type Ⅲ,admitted from Aug. 1994 to Aug 2008 were retrospectively reviewed and followed up for two years. The patients were divided into two groups. Patients in Group A were controls while patients in Group B were treated with high-dose steroids and antibiotics. We evaluated the relation between early complications and age at operation (days), baseline bilirubin, different treatments and 2 year survival. Results 93 patients had early complications,and cholangitis is the most common, There is no statistics difference of complications in age at operation and baseline bilirubin. The complication rate was 63. 6% in group A, and 25. 7% in group B(P＜0. 01). The cholangitis rate was 56. 4% in group A and 23. 9% in group B (P＜0. 01). Two groups showed no significant difference in digestive tract bleeding. The visit frequency rate was 92. 5%, while 2 year survival rate was 51. 2%. Patients with early complications or cholangitis have a 34. 4% or 32. 9% 2 year survival rate, while those without have a 59. 6% or 59. 2%(P＜ 0. 01). Patients with high-dose treatment in group B have a better 2 year survival rate (54. 0% vs 40. 0% ,P＜0. 05). Conclusions Early cholangitis was the risk factor of outcome after Kasai operation. These data implied that the use of high-dose steroids and antibiotics can lower the occurrence of cholangitis and elevate 2 year survival rate.     

胆道闭锁Kasai术后早期并发症的分析

Objective The purpose of this study was to evaluate the relation between early complications of Kasai operation and high-dose steroids and antibiotics and to demonstrate the prognosis of biliary atresia(BA). Methods 281 patients diagnosed with biliary atresia(BA) type Ⅲ,admitted from Aug. 1994 to Aug 2008 were retrospectively reviewed and followed up for two years. The patients were divided into two groups. Patients in Group A were controls while patients in Group B were treated with high-dose steroids and antibiotics. We evaluated the relation between early complications and age at operation (days), baseline bilirubin, different treatments and 2 year survival. Results 93 patients had early complications,and cholangitis is the most common, There is no statistics difference of complications in age at operation and baseline bilirubin. The complication rate was 63. 6% in group A, and 25. 7% in group B(P＜0. 01). The cholangitis rate was 56. 4% in group A and 23. 9% in group B (P＜0. 01). Two groups showed no significant difference in digestive tract bleeding. The visit frequency rate was 92. 5%, while 2 year survival rate was 51. 2%. Patients with early complications or cholangitis have a 34. 4% or 32. 9% 2 year survival rate, while those without have a 59. 6% or 59. 2%(P＜ 0. 01). Patients with high-dose treatment in group B have a better 2 year survival rate (54. 0% vs 40. 0% ,P＜0. 05). Conclusions Early cholangitis was the risk factor of outcome after Kasai operation. These data implied that the use of high-dose steroids and antibiotics can lower the occurrence of cholangitis and elevate 2 year survival rate.     

胆道闭锁Kasai手术效果影响因素的研究进展

胆道闭锁(biliary atresia,BA)是肝内外胆管进行性纤维化引起肝内外胆管硬化和闭锁的一种常见疾病,Kasai手术(肝门空肠吻合术)改善了患儿预后.手术时肝脏的病理状态,包括肝脏纤维化程度、胆管板畸形、肝门部纤维斑块、胆道闭锁类型都对Kasai术后的效果有影响.单纯用年龄评价手术预后有欠妥当.建立胆道闭锁治疗中心以提高外科医师实施Kasai手术的经验有助于改善手术预后,而胆道闭锁脾脏畸形综合征、早产儿、CMV感染对预后有不利影响.激素、熊去氧胆酸及抗生素的预防性使用其有效性目前尚有争议.     

66例胆道闭锁Kasai术后近期疗效分析

目的探讨胆道闭锁Kasai术后的近期疗效及影响生存的因素。方法对2007～2009年本院收治的66例胆道闭锁行Kasai手术的患儿进行回顾性分析。男55例,女11例。手术时年龄30～180d,其中〈60d29例;60—90d20例;〉90d17例。术后3～5d内先后静脉滴注甲基强的松龙20mg、15mg、10mg、5mg,每剂量连用3d后递减,疗程结束后口服强的松每日5mg;熊去氧胆酸每日15mg／kg,口服抗菌素1年。每月检测血总胆红素1次,随访胆管炎是否发作,将是否生存及存活时间、年龄、术后总胆红素水平、胆管炎发作例次等数据录入计算机,进行Kaplan-meier生存分析、Cox regression分析及χ^2检验。结果66例均获随访,随访时间2～24个月。存活46例,死亡20例。Kaplan-meier生存分析显示2年生存率为67．3％,中位生存时间为17．9个月;Coxregressin分析提示血总胆红素为影响生存的因素;Kaplan-meier生存分析显示,术后长期血总胆红素〈34．2μmol／L者2年内生存率最高（P〈0．05）,全组胆汁排出率85％,黄疸完全消退率62％,手术年龄〈60d者术后黄疸消退率为86％,较其他年龄组高（P〈0．05）,胆管炎发生率为30．3％（20／66）。结论影响胆道闭锁Kasai术后近期疗效的主要因素在于术后血总胆红素水平,保持术后血总胆红素〈34．2μmol/L者预后较好。     

胆道闭锁Kasai术后生存率及条件生存率分析

目的通过分析胆道闭锁的近期和远期条件生存率,探讨影响患儿术后生存状况的因素,为后续治疗提供依据。方法选择本院2005-2012年收治的胆道闭锁患儿218名,收集其诊疗过程及随访情况,对其生存率采取Kaplan-Meier生存分析;对不同时间点一段时间内生存情况采用条件生存率分析。结果患儿术后1年、3年、5年自体肝累积生存率分别为83.0%、56.8%、40.2%;术后1年、2年、3年生存至第5年的条件生存率分别为48%、54%、71%;术后1年、2年、3年的1年条件生存率分别为90%、76%、76%。结论胆道闭锁患儿Kasai手术后2~2.5年为远期生存率的转折"里程碑"时间点,术后12个月和24个月为近期生存率的"低谷期"时间点。     

胆道闭锁Kasai术后相关预后因素分析和对策

目的 探讨影响胆道闭锁(biliary atresia,BA) Kasai术后预后的相关因素,了解我省BA的治疗现状.方法 回顾性分析2009年1月至2015年12月间,在山西省儿童医院行腹腔镜探查、胆道造影确诊胆道闭锁后行Kasai术且随访资料完整的91例患儿.采用Kaplan-Meier法计算各亚组患儿自体肝存活率.各亚组之间自体肝存活率的比较应用Log-rank检验,多因素分析采用COX回归模型.结果 全组患儿6个月、1年、2年的自体肝存活率分别为76.9％(70/91)、48.3％(44/91)、36.3％(33/91).按手术日龄将患儿分为:＜60 d(34例)、60～90(含60)d(47例)、90～120(含90)d(8例)和≥120 d(2例)组,各组2年累计自体肝存活率分别为55.8％(19/34)、44.7％(21/47)、12.5％(1/8)和0(P=0.047).男、女童2年累计自体肝存活率分别为36.4％(16/44)和46.8％(22/47),差异无统计学意义(P=0.313).有胆管炎发作组(67例)与无胆管炎发作组(24例)的2年累计自体肝存活率分别为31.3％(21/67)和62.5％(15/24)(P=0.011);黄疸消退明显组(61例)和黄疸消退不明显组(30例)的2年累计自体肝存活率分别为50.8％(31/61)和23.3％ (7/30) (P=0.012);肝功能恢复良好组(53例)和肝功能恢复较差组(38例)2年累计自体肝存活率分别为56.6％(30/53)和21.1％(8/38)(P=0.01);Ⅰ型、Ⅱ型、Ⅲ型闭锁的2年累计自体肝存活率分别为77.8％(7/9)、66.7％(4/6)和31.6％(24/76)(P=0.023),差异均有统计学意义.结论 手术日龄、有无胆管炎发作、黄疸消退情况、肝功能恢复情况及胆道闭锁分型为影响Kasai手术预后的影响因素,其中手术日龄、黄疸消退和肝功能恢复情况是影响生存的独立预后因素.提高民众对胆道闭锁的认知水平,加强术后随访评估,对提高胆道闭锁生存率有重要意义.     

胆道闭锁Kasai术后超声随访的应用价值

目的综合分析胆道闭锁Kasai术后的超声图像,明确超声检查对于临床随访的应用价值。方法回顾性分析2014年3月至2015年9月在本院进行随访的胆道闭锁患儿超声检查结果,对部分阳性图像及参数与天冬氨酸转移酶-血小板比值(APRI)进行比较,根据黄疸消退情况进行分组,分析各组间超声随访指标的差异。。结果不均质肝脏的APRI[0.36(0.21~0.65)]明显高于均质肝脏[0.11(0.06~0.14)]和欠均质肝脏[0.19(0.12~0.32)],P0.05;包膜欠光整和包膜高低不平患儿的APRI[分别为0.33(0.18~0.58)]和0.44(0.27~0.67)],明显高于包膜光滑者[0.19(0.10~0.31)],P0.05;肝脏半定量评分与APRI之间呈正相关,并且有统计学意义(ρ=0.42,P0.05)。肝内胆管囊性扩张患儿APRI[0.46(0.35~0.65)]高于阴性患儿[0.23(0.13~0.42)],两者差异有统计学意义(P0.05)。术后时间6~12个月及≥12个月组中,肝静脉衰减指数(HVDI)与APRI之间均具有明显的正相关性(ρ分别为0.65及0.49,P0.05)。脾肿大患儿APRI[0.33(0.19~0.57)]高于脾脏大小正常患者[0.14(0.09~0.21)],P0.05。黄疸消退C组的肝脏评分[5(4~7)]明显高于A组和B组(P0.05);B组和C组的脾肋下长度[34.9(20~45)mm,35(23~40)mm]均明显大于A组[18(0~30mm)],P0.05。C组中的脾肿大、腹水阳性、单相波比例均明显大于A组(P0.05),C组的三相波比例明显小于A组(P0.05)。结论将肝实质回声、肝表面包膜进行半定量评分可用于评估肝纤维化和手术预后;肝内胆管囊状扩张者肝纤维化程度会更为严重;对于术后6个月以上的患儿,肝静脉呈单相波或波形平坦的双相波对于提示肝硬化具有参考价值;脾肿大(特别是脾肋下长度≥20 mm)和患儿的手术预后较差相关。     

胆道闭锁Kasai术后胆管炎的诊治及预后研究

目的 总结胆道闭锁Kasai术后胆管炎的诊治经验,并分析其长期预后.方法 采用回顾性研究方法,收集2008年6月至2017年6月首都医科大学附属北京儿童医院普外科收治的66例胆道闭锁Kasai手术后胆管炎患儿临床资料,根据初次胆管炎发作时初始治疗用药情况分为头孢哌酮舒巴坦组(17例)、美罗培南组(12例)以及亚胺培南西...     

Focal nodular hyperplasia in biliary atresia patient after Kasai hepatic portoenterostomy

We present the case of a 10-year-old girl with biliary atresia in whom focal nodular hyperplasia (FNH) of the liver occurred following successful “Kasai” hepatic portoenterostomy at the age of 2 months. Her jaundice completely disappeared a few weeks after the operation. A 4-cm diameter liver tumor in segment IV was found when she was 5-years old. Needle biopsy could not establish a definitive diagnosis. Because the tumor size was not changing, she was conservatively followed by abdominal echo at an outpatient clinic. She showed progressive liver dysfunction and ongoing cirrhosis, and so at the age of 10 years, she received living donor liver transplantation at our institute. The tumor was pathologically diagnosed as a FNH. Though a FNH in biliary atresia patients is extremely rare, there are only two cases reported in the literature; it should be considered in the differential diagnosis for a hepatic nodule during a long follow-up course in patients with biliary atresia.     

胆道闭锁Kasai手术后综合管理

胆道闭锁(biliary atresia,BA)是婴儿期肝内外胆管进行性炎症及肝纤维化的疾病,其病因及疾病进展机制尚不清楚.Kasai手术(Kasai portoenterostomy,KPE)是治疗胆道闭锁的首选术式,成功的Kasai手术能够重建胆汁引流,但Kasai手术后长期自体肝生存(native liver s...     

A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy

A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group of those who died or received liver transplantation. A score was calculated that assessed nine factors, including laboratory values and complications. The data were assessed at the time complications occurred. The scores were analysed by a trend analysis to see if serial scores predicted the evolution of liver disease. A receiver operating characteristic (ROC) curve was plotted to assess the optimal cut-point for the scoring system. There were 98 patients in the non-transplantation group and 35 in the failure group. The latter group had significantly higher post-operative bilirubin (9.3±7.2 mg/dl versus 3.5±3.1 mg/dl), ALT (136±89 U/l versus 92±88 U/l), prothrombin time, and incidence of cirrhosis, ascites, oesophageal varices, portal hypertension, cholangitis and sepsis than the non-transplantation group ( P <0.05). A score of 8 had a high sensitivity (96.9%) and specificity (89.5%) for predicting the need for liver transplant. Conclusion:based on easily available clinical information, our scoring system can predict which patients with biliary atresia who have already undergone a Kasai procedure should be considered for liver transplantation.Abbreviations EHBA extrahepatic biliary atresia - ROC receiver operating characteristic     

肝门肠吻合术治疗胆道闭锁112例报告

目的 评价肝门肠吻合术(Kasai手术)治疗胆道闭锁的效果并总结治疗经验.方法 对2007年1月至2011年12月集中收治并采用统一的治疗方案进行Kasai手术的Ⅲ型胆道闭锁病例进行回顾性分析.结果 进行Kasai手术的126例有112例(89.7％)获得完整随访.术后有70例黄疸清除,总黄疸清除率为62.5％,黄疸清除率在手术年龄≤60 d、60～90 d(包含90d)、90～120 d、＞120 d组分别为72.0％、80.5％、60.0％和19.0％,组间差异有统计学意义(P＜0.05).总胆管炎发生率为30.4％(34/112),黄疸清除组(15例)的胆管炎发生率(21.4％)低于未清除组(19例,45.2),组间差异有统计学意义(P＜0.05).术后68例(60.7％)出现持续肝纤维化,在各因素中分组中手术年龄＞90 d、黄疸未清除和胆管炎发作的病例,出现持续肝纤维化的比率较高(分别为93.5％,100％和50％),组间差异有统计学意义(P＜0.05).本组1年和2年自体肝生存率分别为64.9％和61.8％.单因素生存分析发现,患儿手术年龄、术后黄疸消退与否、胆管炎发作与否以及肝纤维化活动情况是影响自体肝生存的相关因素(P＜0.05).结论 术后黄疸清除和胆管炎发作与否以及自体肝生存时间是胆道闭锁Kasai手术的评价指标,手术年龄≤90d,术后黄疸清除和较少的胆管炎发作有利于术后自体肝生存.     

Abdominal malignant lymphoma in a child after successful portoenterostomy for biliary atresia

Malignant disorders in long-term survivors of biliary atresia have rarely been reported. We report a case of abdominal malignant lymphoma in a 5-year-old boy who underwent a successful portoenterostomy for biliary atresia at the age of 45 days. Correspondence to: M. Honzumi     

胆道闭锁肝门肠吻合术手术改进的初步探讨

目的 对胆道闭锁(BA)肝门肠吻合术(PE)进行手术改进并评价治疗效果.方法 对2012年3月至2014年2月进行PE的Ⅲ型BA病例的临床资料进行回顾性分析.本研究病例的PE方式分为扩大肝门部病变切除范围PE手术(EPE)、Nakamura的Kasai改进手术(MKPE)和本研究的改进Kasai手术(OMKPE)3种并根据PE方式将病例分组.对病例的术前临床资料、术后黄疸清除率、胆管炎发作率和自体肝生存率按PE方式分组比较,并按不同手术年龄(≤90d和＞90d两组)再进行组间比较.结果 87例获得完整随访,其中EPE 34例,MKPE 19例,OMKPE 34例.各组手术年龄、体重、术前肝功能指标、肝外胆管Ohi亚型和术中肝纤维化Weerasooriya分级比较均无统计学差异(P＞0.05).OMKPE的术后黄疸清除率和自体肝生存率为73.5％和73.5％高于EPE的38.2％和38.2％ (P＜0.05),但MKPE的为57.9％和58.9％,与OMKPE和EPE比较,差异无统计学意义(P＞0.05),其中≤90 d手术年龄组中OMKPE的术后黄疸清除率和自体肝生存率为90.9％和90.9％以及MKPE的为90％和90％,高于EPE的46.2％和38.5％(P＜0.05),但OMKPE与MKPE比较,差异无统计学意义(P＞0.05),＞90 d手术年龄组中OMKPE的术后黄疸清除率和自体肝生存率为65.2％和65.2％,高于MKPE的22.2％和22.2％以及EPE的12.5％和12.5％ (P＜0.05)但MKPE与EPE比较,差异无统计学意义(P＞0.05).胆管炎发作率各组比较差异无统计学意义(P＞0.05).结论 对比EPE和MKPE,OMKPE手术更有利于提高BA术后黄疸清除率和延长自体肝生存,尤其适用于＞90 d大龄患儿.     

A prospective controlled trial of probiotics on cholangitis after Kasai procedure for biliary atresia

目的 探讨益生菌对胆道闭锁(biliaty atresia,BA)术后胆管炎的预防作用.方法 采用前瞻、随机、对照的研究方法,对60例获得知情同意的Ⅲ型BA术后患儿按照手术后是否辅以益生菌治疗随机分为实验组(30例)和对照组(30例).测定患儿血清总胆红素(TB)、直接胆红素(DB)、丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(AST)水平,结合术后胆汁排出率和随访获得的黄疽消退率及胆管炎发生率综合评定益生菌对胆道闭锁术后胆管炎的预防作用.结果 实验组和对照组患儿平均手术年龄为(52.75±34.30)d和(55.87±29.23)d,手术前各项血生化指标TB(131.49±41.40)μmol/L和(120.16±47.68)μmol/L、ALT(161.01±30.06)μmol/L和(150.21±36.24) μmol/L经统计学分析差异均无统计学意义(P＞0.05),术后2周时两组胆汁排出率为93.3％和90.0％,差异无统计学意义(P＞0.05).随访结果表明术后3个月、6个月时实验组与对照组的胆管炎发生率为58.6％和62.1％、52.0％和54.2％,疗效指标差异均无统计学意义(P＞0.05).结论 益生菌对胆道闭锁术后胆管炎的发生无明显预防作用.     

Kasai术后肝内胆管囊性扩张的诊治与预后

目的 总结胆管闭锁术后肝内胆管囊性扩张的诊治经验,分析其对患儿预后的影响.方法 1998年6月至2008年3月,对胆管闭锁行葛西手术的患儿进行追踪随访.通过超声检查,发现8例患儿存在肝内胆管扩张.其中3例再行MRI检查,6例再行CT平扫加增强检杳以明确诊断.此8例患儿术后均有不同程度胆管炎症状.5例行PTCD显示肝内胆管囊性扩张.3例患儿行囊肿与空肠胆支再吻合术,2例患儿仪行PTCD置管引流,2例患儿暂未予任何处理.结果 3例经手术治疗的患儿,术后黄疸消退或减轻.仅行PTCD的患儿肝内胆管扩张长期存在.结论 对反复发作的胆管炎应定期行超声检查,尽早发现肝内囊肿;对肝内胆管囊性扩张,无论其影像学分型如何,应根据其具体临床表现进行相应的积极治疗;PTCD无法长期放置,仅是暂时性的治疗,但对手术时寻找囊肿有指示作用,使手术时囊肿的定位相对容易;囊肿的大小、位置与门静脉的关系,压迫的时间、治疗是否及时有效都可影响患儿的预后.